Annotation Detail

Information
Associated Genes
PRKAG2
Associated Variants
PRKAG2 p.Arg384Thr (p.R384T) ( ENST00000287878.9, ENST00000392801.6, ENST00000418337.6, ENST00000492843.6, ENST00000650858.1, ENST00000651378.1, ENST00000651764.1, ENST00000652047.1, ENST00000652159.1, ENST00000652321.2 )
PRKAG2 p.Arg384Thr (p.R384T) ( ENST00000287878.9, ENST00000392801.6, ENST00000418337.6, ENST00000492843.6, ENST00000650858.1, ENST00000651378.1, ENST00000651764.1, ENST00000652047.1, ENST00000652159.1, ENST00000652321.2 )
Associated Disease
lethal congenital glycogen storage disease of heart
Source Database
ClinVar
Description
NM_016203.4(PRKAG2):c.1151G>C (p.Arg384Thr) AND Lethal congenital glycogen storage disease of heart
ClinVar Allele ID
181278
ClinVar RefSeq Alternation Syntax
NM_016203.4:c.1151G>C
ClinVar RefSeq Alternation Syntax
NM_001040633.2:c.1019G>C
ClinVar RefSeq Alternation Syntax
NM_001304531.2:c.428G>C
ClinVar RefSeq Alternation Syntax
NM_001363698.2:c.779G>C
ClinVar RefSeq Alternation Syntax
NM_001304527.2:c.776G>C
ClinVar RefSeq Alternation Syntax
NM_024429.2:c.428G>C
Clinical Significance Description
Pathogenic
Clinical Significance Last Update
2007-10-01
Clinical Significance Review Status
no assertion criteria provided
URL
https://www.ncbi.nlm.nih.gov/clinvar/RCV000162026
ClinVar Disease
Lethal congenital glycogen storage disease of heart
Observed Origin Sample
germline
Pubmed
17667862
Drugs